As formas herdadas de HLH normalmente envolvem genes que fornecem instruções para proteínas que ajudam a destruir ou desligar as células imunológicas ativadas quando elas não são mais necessárias. Eles são transmitidos de forma autossômica recessiva. A identificação precoce desta doença é crucial, pois apresenta altas taxas de morbidade e mortalidade, se sub-reconhecida.
A utilidade clínica deste painel é:
George M. R. (2014). Hemophagocytic lymphohistiocytosis: review of etiologies and management. Journal of blood medicine, 5, 69–86. https://doi.org/10.2147/JBM.S46255
Al-Samkari, H., & Berliner, N. (2018). Hemophagocytic Lymphohistiocytosis. Annual review of pathology, 13, 27–49. https://doi.org/10.1146/annurev-pathol-020117-043625
Stalder, G., Ribi, C., & Duchosal, M. A. (2018). Les lymphohistiocytoses hémophagocytaires [Hemophagocytic Lymphohistiocytosis]. Praxis, 107(16), 902–911. https://doi.org/10.1024/1661-8157/a003045
Skinner, J., Yankey, B., & Shelton, B. K. (2019). Hemophagocytic Lymphohistiocytosis. AACN advanced critical care, 30(2), 151–164. https://doi.org/10.4037/aacnacc2019463
Morimoto, A., Nakazawa, Y., & Ishii, E. (2016). Hemophagocytic lymphohistiocytosis: Pathogenesis, diagnosis, and management. Pediatrics international : official journal of the Japan Pediatric Society, 58(9), 817–825. https://doi.org/10.1111/ped.13064
Henter, J. I., Horne, A., Aricó, M., Egeler, R. M., Filipovich, A. H., Imashuku, S., Ladisch, S., McClain, K., Webb, D., Winiarski, J., & Janka, G. (2007). HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatric blood & cancer, 48(2), 124–131. https://doi.org/10.1002/pbc.21039
